Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature

Figure 1 Imaging examinations. A: Endoscopic view of the descending part of the duodenum showing spots of lymphangiectasia suggestive for lymphedema; B: Video capsule endoscopy with a snowflake appearance of the jejunum compatible with dilated mucosal lymphatic vessels; and C: Double-balloon enteroscopy of the jejunum with an almost identical image to video capsule endoscopy.

Figure 2 Histological and immunohistological analyses. A: Jejunal biopsies showing a mild and focal blunting of the villi in particular above the prominent ecstatic mucosal lymph vessels (4-fold magnification); B: Ecstatic lymph vessel without inflammatory changes or abnormalities of the epithelial intestinal cell lining (200-fold magnification).

Figure 3 Histological and immunohistological analyses. A: Periodic Acid-Schiff staining did not reveal any collections of periodic acid-schiff positive histiocytes or inclusions, demonstrating the perfectly normal intestinal brush border (200-fold magnification); B: Immunohistochemistry for D2-40, a marker of lymphatic endothelial cells, confirms the endothelium to be of lymphatic origin and highlights the presence of multiple ecstatic lymph vessels in both the mucosa and submucosa (100-fold magnification). 

Figure 4 Proposed algorithm for diagnosis and therapy of primary intestinal lymphangiectasia in adults.  ¹Including intubation of the terminal ileum. ²Other secondary causes include: Retroperitoneal fibrosis following radio-/chemotherapy, sarcoidosis, intestinal tuberculosis, systemic sclerosis, human immunodeficiency virus-related enteropathy, Fontan-surgery.